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Isabella Nicole Kurek
2006 & 2007
Once again, time seems to have
escaped us; it has been one and a half years since our
last update. Isabella’s condition has remained
incredibly stable, while around her, life has changed in
many ways. When I read the earlier parts of her story,
I am surprised by the angry and bitter tone of the
words. Understandably, those were the emotions we were
feeling at that point in our lives. We were consumed by
the single minded focus to bring Isabella, and
ourselves, through the crisis we found ourselves in.
Now, life seems to have reached a more comfortable
equilibrium and some of the frustrations have eased. I
hope the tone of this portion of her story will reflect
that.
Our first major improvement
occurred a few months after our story left off, in the
spring of 2006. I was able to trade-in my tiny station
wagon for a slightly-used minivan. With all the
traveling back and forth to doctors’ appointments, the
van soon became invaluable. Isabella has always enjoyed
traveling and loves her new ride. Her modified stroller
folds and fits perfectly in the back and there are
plenty of power outlets to feed her ventilator and
suction machine. For the first time we have all been
able to travel in comfort and convenience.
We began seeing a pediatric
orthopedist in March of 2006. Their offices were a part
of the Connecticut Children’s Medical Center (CCMC) in
Hartford. Originally, we only went there to monitor
Isabella’s scoliosis, but we were soon introduced to two
additional departments which proved to be very useful:
orthotics and PT. Orthotics fit Isabella for a thoracic
brace which she wears when she is sitting for extended
periods of time. We thought she wouldn’t like wearing
it, but she actually does! At one of our visits to the
CCMC we were encouraged to visit Scott Vanepps in the PT
department to discuss a power wheelchair for Isabella.
Given our lousy insurance benefits and the $30,000 price
tag of the chairs, we went to the appointment with
trepidation. We knew we would love the chair but we
were afraid we would never be able to afford one. Scott
had a demo chair he allowed Isabella to “test drive” and
she fell in love with it (and Scott) immediately. The
smile on her face when she drove for the first time was
one we will never forget. It was amazing to see a two
year old driving all by her self. After two years of
being completely reliant on others, she was so excited
to be able to move on her own. Regardless, we left the
appointment discouraged. We were teased by the
possibilities and mocked by the financial realities.
Scott assured us the wheelchair companies were very
proactive in securing financial support for families in
need and it turns out he was right! Nine months later,
out of the blue, we got a call telling us that a chair
had been found for Isabella and that money had been
donated to cover it. It was delivered to her shortly
before Christmas of 2006. Santa came through in a BIG
way.
In July of 2006 Isabella was
evaluated by the speech pathology department at Southern
Connecticut State University in New Haven. She spent
the day working with many different speech programs and
devices. Her results showed she was best suited to use
the DynaVox unit, an amazing hand held device activated
with finger switches. Again, the $6,800 price tag was
too much to swallow. Based on these recommendations,
our Birth to Three program was generous enough to
provide Isabella with a slightly scaled down
communication device consisting of her very-own lap top
computer outfitted with Speaking Dynamically Pro.
Looking back, it was a great choice. She not only uses
the computer to assist with her communication, but she
also uses it to surf the internet… something she
discovered all by herself, much to our surprise. We
didn’t even realize she had a wireless connection until
we found her playing on pbskids.com one day. Now she is
a computer wiz, zipping around through her games and
programs using a small roller-ball mouse with one hand
and a “left click” switch with the other. Yet another
reason the computer was a better choice for Birth to
Three, shortly after receiving it, our friends at CCMC
donated a DynaVox to Isabella through an outside
charity. Now she has the best of both.
The next incredible gift to fall
into our lap arrived complements of the Make a Wish
Foundation. Isabella was nominated for a wish by our
nurse, Michelle, in July of 2006. We met with the
foundation and chose a Disney vacation as Isabella’s
wish. After extensive planning and red-tape, her wish
was granted in April of 2007. We were driven to the
airport in a stretch limo and flown to Florida. There,
we spent a week at “Give Kids the World”, a
special-needs resort, outside Orlando. We visited
Disney World, Animal Kingdom, and Sea World. Isabella’s
favorite part of the trip was a special visit from all
of the Princesses. We all had a wonderful time and were
impressed by the thoughtfulness and care shown by the
Make a Wish Foundation and the staff of the resort. It
was a trip we will always remember.
Over the last year and a half,
Isabella has actually traveled quite a bit. In addition
to our Florida vacation, we have driven to Maine twice
to spend time with family. While there, Isabella
enjoyed her first whale watching excursion off the coast
of Bar Harbor, explored the trails of Moose Point State
Park, and luxuriated in the peace and quiet of a
comfortable home in the woods. For the last two years
we have also traveled to the yearly Connecticut SMA
Picnic held at Camp Harkness (a beautiful handicapped
accessible park on the northern shore of Connecticut)
and the SMA benefit walk held in Hamden, CT. Both
events are always a lot of fun and a great opportunity
to see old friends.
One of the biggest changes for
Isabella this year was her transition from Birth to
Three into the Norwalk Public School system. We had
been very happy with her care from Birth to Three and
were worried about the school system’s ability to
maintain it. Looking back, we have had some ups and
downs, but overall we are happy with the transition.
She now receives less hours of PT, OT, and speech
therapy compared to before, but it would be unrealistic
to expect more. In September of 2007, Isabella started
preschool at Kendall Elementary in Norwalk. She spends
half a day, three days a week, and couldn’t be happier.
She loves riding the school bus and interacting with all
the other children. Most days she is accompanied by her
nurse, but occasionally I am able to take her instead.
While enrolled in school, she receives all of her
therapies in the classroom; we no longer benefit from
home visits. For now, we are thinking of keeping her
home through the winter, when the risks of infection
will be their worst. We hope for a full year of
kindergarten next year. The Norwalk Public School
system had a beautiful, brand new manual wheelchair just
the right size for Isabella. The chair had been
purchased for another student but was never needed.
They gave it to Isabella and are allowing her to keep it
for as long as she is in the system. She can even use
it through the periods when she is not attending school
and through the summers. This chair quickly replaced
her modified handicapped stroller (which she was fast
out-growing) as our primary mode of transportation when
we take her out and about. Unfortunately, our van still
hasn’t been modified to accommodate her power chair, so
we are unable to travel with it. Luckily, this new
manual chair has left us with an alternative.
Once Isabella’s power wheelchair
arrived, we soon realized how badly the house needed a
ramp. Without one, the wheelchair was of little use.
As much as Isabella enjoyed driving around the living
room, we all looked forward to the day she could travel
beyond the confines of our home (and, hopefully, one
day, into the van). Once again, the generosity of our
friends and the community helped us when we needed it
the most. In the summer of 2007, Isabella received a
grant from The Angelo James Magliocco Foundation, a
local charity providing support to families with SMA.
This money, along with that collected by our friend,
Adrien Zap, and her “Waves of Hope” long-distance swim
races, helped to finance the materials for the ramp.
The labor was very generously donated by our nurse,
Michelle, and her family.
Our struggle with the insurance
company is one thing that has remained constant. In
March of 2007 we were placed into collections by our
homecare provider, Apria, for over $36,000. This was a
surprise, given the fact that we had never received a
bill from them… just a call straight from the collection
agency. It turns out Apria was billing the insurance
companies and when they stopped paying, the account was
placed in collections under our name. Apria was
charging nearly $2,200 each month for the rental of two
ventilators. When the insurance benefits became
exhausted, the costs fell to us. The problem was that
Apria allowed almost two years of billing to accumulate
before ever notifying us we were being charged. In
order to maximize our insurance benefits, we learned
very early on to purchase our own suction machines,
feeding pumps, and nebulizers. The only medical
equipment we need supplied is the ventilator. And
still, the benefits don’t cover it. When we realized we
wouldn’t be able to afford to rent two ventilators, we
began looking to buy our own.
Our friends at the CCMC
suggested we visit an equipment exchange service in
Hartford called the NEAT Market (New England Assistive
Technology Marketplace). Originally, we visited them
looking for a replacement battery for Isabella’s power
chair. Not only did we find a battery, but we also came
across a treasure chest of suction catheters and a brand
new nebulizer. Just on a whim, I asked the staff if
they ever get any ventilators donated. The gentleman
answered that in eight years of working there, not a
single ventilator had been seen. Not surprised, we
moved on to look for a box to pack up our catheters.
Then, in a moment right out of a storybook, we heard the
gentleman say “Guess what I just found!” while rummaging
through some empty cardboard boxes. We thought maybe
another box of catheters?... but as he emerged from the
rubble holding up a ventilator, our jaws fell to the
floor. Not only did he have a new ventilator, but it
was the exact same model Isabella currently uses. We
were looking through an area of recently donated items
that had yet to be inventoried or priced. Nobody knew
the vent was even there yet. Since it didn’t have a
price assigned to it, the gentleman asked, “How does
$100 sound?” It sounded too good to be true. We
collected our goodies, paid our hundred dollars, and got
out of there as fast as we could. To this day, we still
can’t believe how lucky we were for that ventilator to
fall into our laps. We are now able to return one of
the $1,100/month vents and use our own as the backup.
Finding someone to service a privately owned ventilator
has opened up a whole new can of worms, but that story
is still unfolding.
Despite our miracle ventilator,
we were still left holding a $36,000 tab, compliments of
Apria. We contacted the Child Advocate for the State of
Connecticut, a government office protecting families
against the powerful insurance companies and homecare
providers. The office mediates disputes and encourages
insurance companies to work with families and providers
in an effort to achieve what is best for the patient
(imagine that). They have proven to be an incredibly
helpful and sympathetic resource. By working with the
Advocate we resolved our debt to Apria. Their office
also helped us to maximize our nursing coverage by
modifying the insurance company’s definition of a
“shift” from 4 hours to 12 hours. The Child Advocate
continues to be an incredible source of support for
Isabella and our entire family.
Through the last one and a half
years, Isabella’s health has remained fairly stable. We
are very happy to report that since her initial
discharge from Yale, we have had no need for additional
hospitalization. We continue to see both her
pulmonologist and gastroenterologist every 4-6 months on
a recheck basis. The gastroenterologist monitors her
weight and adjusts her formula and water intake. The
pulmonologist helps us fine-tune the dosing of her
multiple inhalant medications and modifies her vent
settings based on yearly sleep studies performed at
Yale. Although Isabella has been able to avoid any
further episodes of pneumonia, her chronic, fluctuating
tracheitis has remained our largest medical problem.
Since her trach was placed over three years ago she has
been struggling with this on and off, low-grade
infection. She alternates between antibiotics based on
periodic cultures and is very rarely without some sort
of treatment.
Our one and only trip to the
emergency room with Isabella had nothing to do with her
respiratory condition. One day in October of 2007, she
suddenly seemed very painful around her left leg. This
was unusual because of her two knees, the right one had
always been the one that had less range of motion and
was the quickest to become stiff and sore. We knew from
previous x-rays that Isabella’s hips were very weak from
the lack of use. We were concerned that she had
possibly dislocated the left side. We took her to the
emergency room that morning and were surprised to find
that she had actually fractured her left thigh bone,
just above the knee. With absolutely no history of
trauma, we were left wondering how this could have
happened. Again, we understand her bones are weak and
fragile from disuse, but the break still seemed
unusual. Initially, the doctors wanted to transfer
Isabella to Yale for possible surgery, but we felt that
was too aggressive. Isabella is never going to walk and
a surgical repair, with the necessary hospitalization,
was something we felt we could do without. Luckily the
orthopedist agreed and was willing to treat her with a
simple cast. Isabella was such a trooper throughout the
process. She kept her cast on for three weeks before
transitioning to a soft splint for an additional couple
of weeks. At this point, she has completely healed but
we are still left without an answer as to how the break
occurred.
Even though we consider Isabella
medically stable, we have seen slow deterioration of her
strength and stamina. Her ventilator support has
increased and her voluntary muscle movements have
deteriorated. We understand that SMA is a progressive
disease and we expect to see these slow changes.
Overall, we are thrilled with her condition as she
continues to exceed all expectations. When we first
learned of her diagnosis, most of the information
available suggested she wouldn’t live beyond two years.
Obviously devastated, our expectations were low. But
now when we look back at the articles, the statistics,
and the stories of individual children, we see that
these were predominantly about patients that were not
treated “aggressively.” Very few, if any, were trached
and provided with ventilator support. We are certainly
in no position, nor presume to judge the decisions made
by other families affected by SMA. Every patient is
different, as are the family values and circumstances
surrounding that patient. I only wish there had been
more information available regarding the prognosis of
children treated more aggressively. It would have been
comforting to have known then, what we have grown to
know by simple experience, now. I hope Isabella’s story
can serve as a testament and provide a source of support
for families considering their treatment options. As
Isabella’s fourth birthday quickly approaches, we
continue to look to the future with cautious optimism.
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